CASE REPORT

'Moya-moya' Disease Caused by
Cranial Trauma
Emilio Fernandez-Alvarez, MD, Mercedes Pineda, MD,
Carlos Royo, MD, and Ramon Manzanares, MD

A case of "maya-maya" disease of a 12-year-old boy is reported_ The clinical history started at 3
years 2 months after cranial trauma_ The patient developed mental retardation, hemiparesis and
seizures.
Ferntindez-Alvarez E, Pineda M, Royo C, Manzanares R: "Maya-maya" disease
caused by cranial trauma. Brain Dev 2: 133-138,1979

"Moya-moya" disease [8, 9] is a cerebrovascular process characterized by stenosis or occlusion of the arteries of the circle of Willis with
the development of collateral telangiectasic network involving principally the internal carotids,
external carotids, ophthalmic artery, and leptomeningeal vessels.
One of the unanswered questions about
moya-moya disease is its etiology [4]. We have
observed a patient whose clinical and radiological characteristics are of moya-moya disease
and that most probably was caused by cranial
trauma. We think this case might help clarify
the etiology of this syndrome.
Case Report
The patient was a 12-year-old male admitted to
the hospital of San Juan de Dios for observation of symptoms of convulsions and mental
From the Departments of Pediatric Neurology (EF,
MP, CR), Pediatric Radiology (RM), Hospital Infantil
de San Juan de Dios, Barcelona.
Received for pUblication: August 6, 1979.
Accepted for publication: September 18, 1979.

Key words: Moya-moya disease, multiple vascular occlusions, circle of Willis, head trauma, hemiparesis.
Correspondence address: Dr. Emilio FernandezAlvarez, Department of Pediatric Neurology, Hospital
Infantil de San Juan de Dios, Servicio Neurologi'a,
Carretera de Esplugas sIn Barcelona-17, Spain.

retardation (Ref: 72,286). The parents, who are
unrelated, denied the occurrence of related
neurologic disorders in their families previously.
The patient's 5-year-old sister showed slight
mental retardation, but no signs of vascular
anomalies were found when a carotid arteriography was done. The patient's two brothers,
aged 14 and 7 years, were both of normal intelligence.
The patient weighted 3.2 kg at birth after a
normal pregnancy and delivery. His psychomotor development was normal: he could hold
up his head at 5 months, sit alone at 8 months
and walk at 14 months.
At the age of 3 years 2 months, he was
knocked down by a car and he hit his face on
the curbstone of the pavement. He did not
loose consciousness immediately, but he was
hospitalized nevertheless. Three days later a
convergent strabismus appeared in his right eye.
The patient went into a coma in a few hours ,
and remainded in this state for 24 hours, recovering slowly. Convulsions were not observed
during this time . In the following weeks,
aphasia and a right hemiparesis developed. The
patient was able to eat without difficulty six
weeks later.
The patient began physiotherapy at the age
of four years. At that time his right Achilles
tendon and right calf muscle were elongated
due to spasticity. He suffered his first seizures

Fig 1 PEG: The fourth ventricle was slightly dilated (solid arrow). The magna (open arrow) and
interpeduncularis (double solid arrows) cisterns were filled up with air but no air passed to the
sub-arachnoid spaces of the convexity.

134 Brain & Development, Vall, No 2, 1979

Fig 2 Right carotid angiography: Enlarged ophthalmic artery (solid arrow). Network of ethmoidal
arteries (small solid arrowheads). Obstruction of anterior (open arrow) and middle (double open
arrows) cerebral arteries.

at the age of four; it consisted of a right tonicclonic hemiconvulsion with sphincter incontinence. Since then he has received uninterrupted
treatment with phenobarbital, hydantoins, and
diazepoxides. The seizures did not recur until
the age of nine, but since then the patient has
had right-sided tonic convulsions wit,!l oculocephalic torsion to the right two or three times
every month. The passage of years has produced mental retardation, and at the age of 11
the patient's IQ was 20 (Terman-Merrill test).
The right hemiparesis has evolved unfavorable,
the retractions and the spasticity continued to
worsen, and growth in the right half of the
body has been retarded.

Clinical Examination
We first saw the patient when he was 12 years
old. His head circumference was normal (54 cm).
There were operation scars in the back of the
calf and in the region of the Achilles tendon of
the right foot , corresponding to the orthopedic
operations. No abnormal pigmentation nor cardiac palpitations were detectable.
Physical examination revealed paresis of the
VI right cranial nerve. Right spastic hemiparesis
with the shoulder turned forward and retraction of the right major pectoralis muscle. The
right arm was held in pronation and flexion.
Hands clawed, and luxation of the thumb. The
right leg showed popliteal retraction with the
foot at right angle. The patient's right hand was

Fernandez-Alvarez, et al: "Moya-moya " disease 135

Fig 3 Right carotid angiography : Shows enlarged ophthalmic artery (solid arrow).

smaller than the left one. The right lower extremity was 0.7 cm shorter than the left, and
the big toe was in a hammered position. There
was right sided hyperreflexia. His vision was
mal. His pupils were isocoric. Blood pressure
was 110/80. The patient was able to understand
only very simple instructions. He knew some
vowels, was very sociable and of a very happy
disposition. He was unable to control his
sphincters.
He was admitted to the Hospital de San Juan
de Dios for observation and study due to an increase in convulsions and spasticity and failure
to progress intellectually.

136 Brain & Development, Vol 1, No 2, 1979

Laboratory Data
Routine laboratory analysis was normal. Normal values were obtained for hemogram, WBC
formula, coagulation tests, urine analysis,
aminoacids, chest X-rays, and CSF (proteins:
13.5 mg%). EEG showed signs of marked disorganization in basic rhythms, but not significant focal activity was observed.
Radiological Data
Plain skull X-ray showed thickening of the calvaria, most marked on the left side. The left
frontal sinus was larger than the right. Neither
calcifications, digital markings nor abnormal
grooves were seen.

Fig 4 Left carotid angiography: Shows the "rete mirabile" of the anterior
two thirds of the calvaria (open arrows).

Pneumoencephalography (Fig 1) showed a
pronounced ventricular dilatation that was
most severe in the frontal area where the distance from the frontal horn to the inner table
was 11 mm. The distance between the occipital
horn and the inner table was of 38 mm. The
fourth ventricle was slightly dilated. The magna
and interpeduncularis cisterns ftIled up with air
but no air passed into the subarachnoid'spaces
of the convexity.
Bilateral carotid and vertebral angiography
were performed (Figs 2 and 3). They rendered
the following findings:
1. Both ophthalmic arteries were enlarged and
feed a large network of ethmoidal arteries
and the distal portion of the anterior cerebral artery.
2. The proximal portion of both anterior cerebral arteries was occluded and the distal portion fIlled through collateral circulation. The
pericallosal arteries were thin and stretched
around the dilated ventricles.
3. There was complete obstruction of both
middle cerebral arteries with profuse local
collaterals around the occluded vessels. The
distal portion of the sylviim vessels seemed

to fill through the posterior cerebral arteries.
4. An important network of collateral vessels at
the level of the basal ganglia and through the
rete mirabile (Fig 4) of the anterior two
thirds of the calvaria.

Comment
There is agreement that moya-moya disease
consists of a progressive arterial process in which
the normal collateral circulation of the circle of
Willis is obstructed and telangiectases arise as an
atypical consequence of the arterial occlusion
[6, 11]. However, the etiology of moya-moya
disease is unsettled. The causes seem to be
manifold [4] and genetic factors may be
evoquedin familial cases [2,5,9, 10].
The occurrence of intracranial vascular occlusions after the head injury and the apparent
relation between the trauma and the development of the symptoms in our patient strongly
suggest that cranial trauma was the determining
factor in this case. In spite of the fact that
Suzuki and Takaku [10] reported head injury
in three out of the 20 cases, we were able to
fmd only another report of moya-moya disease

Fernandez-Alvarez, et al: "Maya-maya" disease 13 7

ascribed to cranial trauma [7J . On the other
hand, there are some reports of thromhosis of
the cerebral arteries after cranial or cervical
trauma [1, 3J . The report of Frantzen et al [3]
is of special interest. They observed that cranial
trauma in a child led to occlusion of the right
middle cerebral artery one centimeter from the
bifurcation of the internal carotid. Fifteen
months later, as $ymptoms reappeared, another
occlusion of the anterior cerebral artery appeared on the right side a few millimeters above
the previous one. Their observations as well as
ours suggest that non-penetrating cranial or
cervical trauma in children may cause disorders
in cerebral circulation. Thus, cranial trauma
should be considered in the etiology of this
type of disorder including moya-moya disease.
Summary
A case report of a patient with a clinical history
of hemiparesis, convulsions and mental retardationdating back to cranial trauma is presented.
Angiography showed the characteristics of the
so-called "moya-moya" disease in the patient.
It is argued that cranial trauma could be an
etiological factor in this disease .

138 Brain & Development, Vol 1, No 2, 1979

References
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cerebral artery occlusion. Neurology 13 : 613,
1963.
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Neurologie Infantile, Paris, 1971.
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head and neck. Neurology 11 : 695, 1961.
4. Halonen H, Halonen V, Donner M, et a1: Occlusive disease of intracranial main arteries with
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